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C A S E R E P O R T Open AccessCarbon ion radiotherapy for basal cell adenocarcinoma of the head and neck: preliminary report of six cases and review of the literature Keiichi Jingu*, Az

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C A S E R E P O R T Open Access

Carbon ion radiotherapy for basal cell

adenocarcinoma of the head and neck:

preliminary report of six cases and review of the literature

Keiichi Jingu*, Azusa Hasegawa, Jun-Etsu Mizo, Hiroki Bessho, Takamichi Morikawa, Hiroshi Tsuji, Hirohiko Tsujii, Tadashi Kamada

Abstract

Background: Basal cell adenocarcinoma accounts for approximately 1.6% of all salivary gland neoplasms In this report, we describe our experiences of treatment for BCAC with carbon ion radiotherapy in our institution

Methods: Case records of 6 patients with diagnosis of basal cell adenocarcinoma of the head and neck, who were treated by carbon ion radiotherapy with 64.0 GyE/16 fractions in our institution, were retrospectively reviewed Results: In a mean follow-up period of 32.1 months (14.0-51.3 months), overall survival and local control rates of 100% were achieved Only one grade 4 (CTCAE v3.0) late complication occurred There was no other grade 3 or higher toxicity

Conclusions: Carbon ion radiotherapy should be considered as an appropriate curative approach for treatment of basal cell adenocarcinoma in certain cases, particularly in cases of unresectable disease and postoperative gross residual or recurrent disease

Background

Basal cell adenocarcinoma (BCAC) was first recognized

in 1978 and accounts for approximately 1.6% of all

sali-vary gland neoplasms [1] BCAC typically arises in

adults older than 60 years of age and has no gender

pre-dominance [2] The vast majority of BCACs occur in the

parotid gland (about 90%) [3-5], followed by the

sub-mandibular gland and minor salivary glands [6] The

2005 WHO classification categorizes BCAC as a

low-grade tumor with a favorable prognosis [7] The

stan-dard treatment has been wide local excision with or

without postoperative radiotherapy However, local

recurrence has frequently been reported

Carbon ion radiation therapy (C-ion RT) was initiated

at the National Institute of Radiological Sciences (NIRS)

in 1994 [8] For malignant tumors of the head and neck,

a phase II clinical trial with C-ion RT was started in

April 1997 So far, we have treated more than 350 patients with a large histological variety of malignant tumors of the head and neck including mainly mucosal malignant melanoma and adenoid cystic carcinoma Of those patients, 6 patients with BCAC of the head and neck were enrolled In this report, we describe the 6 patients with BCAC and the efficacy and complications

of C-ion RT

Methods

Case Presentation

The 6 patients’ characteristics are shown in Table 1 Mean age was 58 years (range: 37-81 years) None of the patients had metastasis in distant organs The pri-mary sites were parotid gland in 4 patients, base of the tongue in 1 patient and ethmoid sinus in 1 patient The stages for all patients were defined according to Unio Internationalis Contra Cancrum (UICC) 2002 Histology

of all patients was reconfirmed by a pathologist in our institution before C-ion RT

* Correspondence: kjingu@nirs.go.jp

Research Center for Charged Particle Therapy, National Institute of

Radiological Sciences (NIRS), Chiba, Japan

© 2010 Jingu et al; licensee BioMed Central Ltd This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in

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Clinical Histories

Patient 1

A 43-year-old Japanese male developed a sore throat

over a period of 3 months A tumor at the base of the

tongue was detected by endoscopy The pathological

diagnosis was BCAC by biopsy CT revealed that the

clinical stage was T4aN0M0 (stage IVA) The diameter

of the primary tumor was 29 mm At first, one cycle of

chemotherapy, including cisplatin, 5-FU and docetaxel,

was performed in the previous hospital; however, the

tumor did not show shrinkage He therefore came to

our institution for C-ion RT

Patient 2

A 70-year-old Japanese male had nasal bleeding for one

week A tumor in the right ethmoid sinus was detected

by endoscopy and CT in the previous hospital Biopsy

was performed in the previous hospital, and the

diagno-sis was BCAC (MIB-1 index, 50-80%) in the right

eth-moid sinus with intracranial invasion The diameter of

the primary tumor was 50 mm and there was no

lym-phadenopathy (cT4aN0M0, stage IVA) There was no

indication for surgery He came to our institution for

C-ion RT The patient had bilateral retinal detachments

as a past history

Patient 3

A 62-year-old Japanese female had undergone right total

parotidectomy in the previous hospital (pT3N0M0, stage

III, R0) The pathological diagnosis was BCAC

There-after, follow-up was performed every 3 months Eight

years after parotidectomy, a tumor of 54 mm in

dia-meter was detected under the right temporal skin by

MRI, and BCAC recurrence was confirmed by biopsy

No lymphadenopathy was detected There was no

indi-cation for surgery She came to our institution for C-ion

RT

Patient 4

A 37-year-old Japanese female developed fullness in the

right ear and right buccal swelling over a period of 3

months She underwent fine needle biopsy and was diag-nosed as cytologic class III in the previous hospital Total parotidectomy +/- postoperative radiotherapy was planned CT revealed that the clinical stage was T3N1M0 (stage III) The diameter of the primary tumor was 54 mm and the diameter of the right upper cervical lymph node was 18 mm However, she declined surgery and requested C-ion RT We required the previous hospital to perform biopsy for confirming the histology Thereafter, her tumor was diagnosed as BCAC (MIB-1 index, 10%)

Patient 5

An 81-year-old Japanese male developed left buccal swelling over a period of one and half years A benign tumor was suspected by CT, but the histological diagno-sis was BCAC by biopsy The clinical stage was T4aN0M0 (stage IVA) The diameter of the primary tumor was 52 mm and there was no lymphadenopathy

If curative surgery was performed, facial nerve palsy could not be avoided For this reason, he declined cura-tive surgery and selected C-ion RT

Patient 6

A 55-year-old Japanese male had right buccal swelling

A benign tumor was suspected and observation was per-formed Four years later, a gastric malignant tumor was found by medical examination Right partial parotidect-omy was performed simultaneously with total gastric resection The histological diagnosis of the parotid tumor was BCAC with suspected residual macroscopic tumor (pT4aN0M0, stage IVA, R2) For gastric cancer, chemotherapy including TS-1 was performed for 6 months after surgery However, a gross tumor of 19 mm

in diameter in his right parotid gland remained He selected C-ion RT

Treatment

All of the patients were not indicated for curative sur-gery or declined sursur-gery, and C-ion RT was performed

as follows

Table 1 Patients’ Characteristics

Patient Age Gender Primary

Site

Stage (UICC§ 2002) Tumor Response

(RECIST*)

Grade 3 or more Toxicities (CTCAE † v3.0) Observation Period(months)

tongue

sinus

grand

postoperative recurrence (pT3N0M0, R0)

grand

grand

grand

postoperative residual (pT4aN0M0, R2)

Abbreviation, §Unio Internationalis Contra Cancrum; *Response Evaluation Criteria in Solid Tumors; †Common Terminology Criteria for Adverse Events.

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Carbon Ion Radiotherapy

Doses of carbon ions were expressed in photon

equiva-lent doses (GyE), which were defined as the physical

doses multiplied by the RBE of the carbon ions The

biological flatness of the SOBP was normalized by the

survival fraction of human salivary gland tumor cells at

the distal region of the SOBP, where the RBE of carbon

ions was assumed to be 3.0 [9]

The patients were positioned in customized cradles

(Moldcare; Alcare, Tokyo, Japan) and immobilized with a

low-temperature thermoplastic shell (Shellfitter; Kuraray,

Osaka, Japan) A set of 2.5-mm-thick computed

tomogra-phy (CT) images was taken for treatment planning with

the immobilization devices CT imaging alone is

inade-quate for detection of extension of the tumor Therefore,

MRI was routinely used for identification of the tumor,

after fusing it with the planning CT Determination of

gross target volume (GTV) was based on

contrast-enhanced MRI The clinical target volume (CTV) had

minimum margins of 5.0 mm added around the GTV

The planning target volume (PTV) included margins of

3.0-5.0 mm around the CTV, and this could be modified

manually The PTV and OAR (e.g., eyeball wall, optic

nerve, optic chiasma and brain stem) were outlined on the

planning CT images to permit dose-volume histogram

(DVH) analysis Three-dimensional treatment planning

was performed using HIPLAN software (National Institute

of Radiological Sciences, Chiba, Japan) [10] The PTV was

ensured with at least 95% of the prescription dose

Irradiation was carried out once per day for 4 days per

week (Tuesday-Friday) with carbon ion beams The

pre-scribed dose to the center of the CTV was 64.0 GyE/16

fractions over 4 weeks at 4.0 GyE/fraction per day in all

of the 6 patients Thereafter, no other treatments were

performed for any patients

Follow-up

The patients were followed up by CT or MRI every 1 or 2

months for the first 6 months after C-ion RT and

there-after every 3 to 6 months The overall survival and local

control rates were calculated from the first day of C-ion

RT Toxicities were classified according to Common

Ter-minology Criteria for Adverse Events (CTCAE) v3.0

Results

All of the patients underwent C-ion RT without an

interval, and all of the patients were alive at the last

observation date No patient was lost to follow-up The

mean observation period was 32.1 months (range:

14.0-51.3 months) There were no local or regional

recur-rences or metastasis in distant organs Tumor response

rate according to Response Evaluation Criteria in Solid

Tumors (RECIST) was 66.7%, including 1 CR, 3 PR and

2 SD, at 6 months after completion of C-ion RT MR

images of 2 representative patients before and after C-ion RT and dose-distributions of C-ion RT are shown

in Figures 1 and 2, respectively

One patient who had a tumor in the left ethmoid sinus had grade 4 left retinopathy (light perception) about 12 months after completion of C-ion RT Three

of the 4 patients who had a tumor in the parotid gland did not show facial nerve palsy; however one patient showed slight facial nerve palsy 6 months after C-ion

RT There was no other grade 3 or higher toxicity in the 6 patients

Discussion

Since BCAC seldom metastasizes to cervical lymph nodes, routine neck dissection is not recommended The mortality rate for this tumor is also low, although reported local recurrence rates are high In a review, local recurrence was observed in 37% (17/46) of patients with follow-up between 6 months and 2 years [2] In another review, local recurrence was observed in 44.4% (8/18) of patients with follow-up between 2 years and 14.3 years [11] From the above experiences, it would appear that the first treatment of choice for BCAC is wide local excision with frozen-section control of the resection margins However, sufficient resection margins often cannot to be obtained due to the need for preser-vation of critical organs (e.g., the facial nerve in parotid tumors) Therefore, postoperative radiotherapy has been proposed for lesions with a high risk of vascular and neural invasion and for lesions that are diffusely infiltra-tive, especially in patients with close resection margins [12] Even with wide local excision and postoperative radiotherapy, local recurrence has been reported in about 30-50% of patients (Table 2) [2,11,13] To our knowledge, this is the first report of BCAC treated with radiation alone Although observation period of the pre-sent cases was not enough, C-ion RT achieved good local control among past reports A possible explanation for the success we have seen with C-ion RT of BCAC concerns the differences in biological interactions of car-bon ion radiation and photon radiation in tissue Com-pared to photon radiation, high linear energy transfer (LET) radiation is characterized by less variation of sen-sitivity through the cell cycle [14], by less or no repair

of sublethal or potentially lethal cell damage, which is a problem in controlling repair-proficient photon-resistant tumors, and by a reduced oxygen enhancement factor (OER) in the case of hypoxic and poorly-reoxygenating tumors An indolent tumor such as BCAC with conse-quent ability to repair potentially lethal damage from low LET radiation might have an increased responsive-ness to C-ion RT High LET radiation, including C-ion

RT, could be a favorable curative treatment for BCAC More long-term observation is required

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With regard to toxicities, severe unilateral retinopathy

occurred in one patient (patient 2) even with excellent

dose-distribution of C-ion RT since the critical organ

was next to the tumor We have already revealed the

dose constraints of optic nerves for C-ion RT [15]

Severe retinopathy was considered to be unavoidable in

that patient Grade 3 or more toxicity was observed in

only that patient Brown et al reported that severe facial

nerve palsy occurred in 26% of 66 patients who

under-went surgery even with facial nerve graft for a parotid

neoplasm and postoperative radiotherapy, [16] On the

other hand, Buchholz et al reported that facial nerve

palsy occurred in one of 6 patients with recurrent

pleo-morphic adenoma treated by fast neutron radiotherapy,

which is also high LET radiation [17] Duthoy et al

reported that decrease of vision occurred in 5 of

39 patients with sinonasal carcinoma treated with

postoperative intensity-modulated radiation therapy [18] Compared with those treatment methods, C-ion

RT is considered to be acceptable However, the average time of progression to eventual radiation-induced visual loss was 25.6 months (range, 10-41 months) after C-ion

RT in our previous investigation [15] Although facial nerves are considered to be stronger than optic nerves for C-ion RT since peripheral nerves are known to have more radio-resistance than central nerves [19], more facial nerve palsy in patients with a tumor in the parotid gland may occur in the long term The acceptable dose

of C-ion RT for facial nerves is currently under investigation

Conclusions

We reported preliminary but excellent efficacy of C-ion

RT for BCAC, which is very rare head and neck

Figure 1 Patient 2, a 70-year-old Japanese male with BCAC in the ethmoid sinus (a) Axial contrast-enhanced T1-weighted MR image before C-ion RT, (b) Coronal contrast-enhanced T1-weighted MR image before C-ion RT, (c) Histological findings of HE staining at

low-magnification, (d) Histological findings of HE staining at high-low-magnification, (e) Dose-distribution of C-ion RT in axial and coronal CT images, (f) Axial contrast-enhanced T1-weighted MR image 1 year after C-ion RT, (g) Coronal contrast-enhanced T1-weighted MR image 1 year after C-ion RT.

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malignant tumor, in 6 patients Our results showing

acceptable toxicities and appreciable efficacy suggest

that C-ion RT could be one of the curative primary

treatments of BCAC

Consent

Written consent for publication was obtained from all of the patients before C-ion RT in our institution

Authors ’ contributions

KJ and AH conceived the idea, did the literature search and prepared the manuscript KJ, AH, JM, HB, TM and HT performed treatment and follow-up and acquisition of data TK and HT provided critical review of the manuscript and research guidance All authors read and approved the final manuscript Competing interests

The authors declare that they have no competing interests.

Received: 15 July 2010 Accepted: 4 October 2010 Published: 4 October 2010

References

1 Klima M, Wolfe K, Johnson PE: Basal cell tumors of the parotid gland Arch Otolaryngol 1978, 104:111-116.

Figure 2 Patient 5, an 81-year-old Japanese male with BCAC in the left parotid gland (a) Axial contrast-enhanced T1-weighted MR image before C-ion RT, (b) Coronal contrast-enhanced T1-weighted MR image before C-ion RT, (c) Histological findings of HE staining at

low-magnification, (d) Histological findings of HE staining at high-low-magnification, (e) Dose-distribution of C-ion RT in axial and coronal CT images, (f) Axial contrast-enhanced T1-weighted MR image 3 years after C-ion RT, (g) Coronal contrast-enhanced T1-weighted MR image 3 years after C-ion RT.

Table 2 Literature Review of Treatment Results for Basal

Cell Adenocarcinoma

Author n Observation

Period (mean)

Treatment Local

Recurrence Muller et al.

[2]

7 5-192 months

(54 months)

Surgery +/- X-ray

2/7 Parashar

et al [11]

18 2-14.3 years

(5.1 years)

Surgery +/- X-ray

8/18

Nagao et al.

[13]

10 1-18 years

(6.5 years)

Surgery +/- X-ray

5/10 current

series

6 14.0-51.3 months

(32.1 months)

Carbon ion radiotherapy

0/6

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18 Duthoy W, Boterberg T, Claus F, Ost P, Vakaet L, Bral S, Duprez F, Van

Landuyt M, Vermeersch H, De Neve W: Postoperative intensity-modulated

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doi:10.1186/1748-717X-5-89

Cite this article as: Jingu et al.: Carbon ion radiotherapy for basal cell

adenocarcinoma of the head and neck: preliminary report of six cases

and review of the literature Radiation Oncology 2010 5:89.

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